by Mark Wert, The Cincinnati Enquirer

by Mark Wert, The Cincinnati Enquirer

CINCINNATI - Liam Sprague's road to recovery started when his parents took a calculated risk.

Their decision saved his life, ultimately making him strong enough to be a candidate for a heart transplant. After nearly a year in hospitals (mostly Cincinnati Children's Hospital Medical Center), the boy is now home in Indianapolis. His new heart is doing fine.

But the 4 1/2-year-old's road toward a normal life remains incomplete due to an unwanted but not unexpected detour ‚?? a stroke, a serious complication of the lifesaving pre-transplant treatment. Compound that with the other bump on the road to the transplant: Liam came down with a cancer-like illness so rare in children that his is only the 11th documented case.

The boy's medical odyssey "is a really long story," said his mother, Carolyn. It began last June, when his parents took him to Peyton Manning Children's Hospital in the suburbs of Indianapolis because he was breathing too fast ‚?? a side effect of asthma or allergies, with which he had already been diagnosed.

But the results of the new exam dramatically changed the landscape of Liam's life. He had restrictive cardiomyopathy, doctors said. In an instant, Liam needed a heart transplant.

It was the first rarity of Liam's odyssey. Roughly 12 cases of cardiomyopathy occur in every 1 million children, which translates into roughly 1,000 new cases being diagnosed each year in America.

Restrictive cardiomyopathy accounts for 3 percent to 5 percent of all cases in adults. It is even rarer in children, according to the Children's Cardiomyopathy Foundation. Scientists don't know what causes it in children, although it is the leading cause for pediatric heart transplants.

The condition caused the walls of the lower chambers, or ventricles, of Liam's heart to pump normally but kept them too rigid to relax. As a result, the upper chambers of his heart became enlarged. Over time, the condition restricts blood flow to the heart.

The gravity of Liam's condition was such that on June 26 he went into cardiac arrest. "They even coded him," his mother said.

Liam's parents were running out of options. And time. Once their son was stabilized, they headed to Children's Hospital. Liam's father, Richard, joined Carolyn, who had left her job as an oncology nurse to become a full-time caregiver, on weekends and any other time he could spare away from his job in Indianapolis as a restaurant manager.

As doctors and others at Children's worked to get Liam placed on a list for a donated heart, he was diagnosed with Castleman disease.

Named for the doctor who discovered it in the 1950s, the malady attacks the lymph nodes ‚?? a key part of the body's immune system ‚?? and "supersizes" them. While not officially classified as a cancer, the American Cancer Society notes that it is usually treated the same way as lymphoma (or cancer of the lymph nodes) ‚?? with chemotherapy and radiation. Only 200 cases are diagnosed annually in the U.S., most often in adults who have been exposed to the human immunodeficiency or human papilloma viruses.

After three rounds of chemotherapy and other treatments, Liam's heart needed help ‚?? specifically the Berlin Heart Ventricular Assist Device, an external pump that boosts the natural function of the heart. Doctors at Children's use it five or six times a year to help children whose hearts cannot pump enough blood.

Liam was on the machine longer than any patient ever at Children's.

The problem: Use of any ventricular assist device in adults and children often results in serious complications such as bleeding and infection. Patients on the Berlin machine have a 3 in 10 chance of having a stroke.

For the Spragues, the choice was clear. "If you didn't take the Berlin, you don't have a chance" for Liam to survive long enough to find a donor heart, his mother said on a family visit to Children's last month for tests. "It was the only choice."

Liam responded well to the anti-Castleman treatments and being on the assistive device. "He was weak, but he was him," father Richard said. By Sept. 18, Liam finally made it to the heart transplant list.

On Oct. 1, the next complication set in. This time, it wasn't rare.

The tissue inside Liam's brain was bleeding. It was what the doctors call an intraparenchymal hemorrhage and what laypeople call a stroke.

The stroke was a double misfortune because Liam had to be removed from the transplant list again. But he rallied. By Nov. 26, he was back on the list. "Thank the Lord for answered prayers," his family wrote on Liam's Caring Bridge page.

Liam's odyssey had two more twists. A donated heart had to be rejected at the last minute because the transplant team concluded too much time had passed from being harvested to arriving at Children's. And Carolyn had to leave Liam's side for a welcome "complication." She gave birth to a daughter, Amelia, on Jan. 2 at Christ Hospital.

On Feb. 25, Liam finally received his new heart; he is one of 111 children to receive such a transplant at Children's. On March 13, he was transferred to a rehab unit to continue recovering from the stroke. He progressed and his body showed no signs of rejection. So the Spragues returned to Indianapolis as a family May 1.

They're back at Children's Hospital every other week for tests and to "see almost every -ologist and -ist here," said Richard.

Liam "does a little more every day, much more than he did here in the hospital," his dad said.

He's eating food by mouth "and manages to say 'all done,'" his mother said. His parents take him twice a week for three-hour sessions in physical, occupational and speech therapy. And they wait to see how his recovery from the stroke will unfold.

"You can't fix his brain," his mother said. "He has to relearn things. His brain has to rewire itself."

But they remain hopeful. During their June visit, they dressed him in a Superman shirt. Because that's how they see him.